Heme Onc

Question 1

incr: aPTT, bleeding time, and Ristocetin cofactornorm: platelets, PT

Answer 1

vWF deficiency (binds factor VIII)

Question 2

tx for vWF deficiency? If hemorrhaging?

Answer 2

DDVAP aka desmopressin

Cryoprecipitate for hemorrhage

Question 3

this OTCRx makes vWF defiency much worse?

Answer 3

aspirin

Question 4

Hemochromatosis

Answer 4

Too much iron absorption in the gut.

Secondary to genetics, alcoholism, or excess blood transfusion

“bronze diabetes”, CHF, micronodular cirrhosis

see: Incr ferretin, transferrin saturation decr: TIBC

Question 5

Hemochromatosis patients need screened regularly using this tumor marker

Answer 5

aFP

hepatocellular CA

Question 6

porphoria cutanea tarda

Answer 6

deficiency in uroporphinogen decarboxylase (part of heme synth pathway)

see: painless hand blinsters, skin hyperpigmentation, facial hair (“hypertrichosis”)

can be triggered by EtOH, estrogens

dx: increased urine porphyrin levels
do: phlebotomy, hydroxychloroquine

if co-infected with Hep C give INFa

Question 7

DIC

Answer 7

happens after significant risks: trauma, burns, sepsis, fetal demise, amnio fluid embolus

labs: incr PT, PTT, finbrinogen split products, d-dimer
decr: fibrinogen, platelets

do: FFP if platelets

Question 8

PT

Answer 8

extrinsic pathway (Factor VII only)

Question 9

PTT

Answer 9

intrinsic pathway (Factor 8,9,11,12)

Question 10

This disease has an abnormal proliferation of plasma cells that produce copious amounts of IgG, IgA

Answer 10

Multiple Myeloma

Question 11

Symptoms of Multiple Myeloma

Answer 11

bone pain, pathologic fractures, hat size increasing
hypercalcemia

CRAB = hyperCalcemia, Renal prob, Anemia, Bone pain

Question 12

Labs of Multiple Myeloma

Answer 12

1) hyperuricemia (2 to plasma cell turnover)
2) anemia (making plasma, not much else)
3) incr BUN and creatinine (immunoglob gunks up kidney)
4) hypercalcemia (lytic lesions)
5) high total protein, normal albumin

Question 13

Fun fact about Bence-Jones proteins

Answer 13

They are not picked up by urine dipstick; they only detect albumin. 24H urinalysis will show elevated proteins 2 to Bence-Jones bc this uses urine immunoelectrophoresis

Question 14

Most accurate test to dx Multiple Myeloma

Answer 14

Bone marrow biopsy

see greater than 10% plasma cells

Question 15

Vitamin K dependent clotting factors

Answer 15

2, 7, 9,10

Question 16

Thrombocytopenia, renal failure, hemolytic anemia, paired with fever and AMS

Answer 16

TTP
may be idiopathic, 2ndary to drug toxicity, or HIV

do: peripheral smear, look for schistocytes
tx: “plasma exchange”